Acquired Haemophilia – a study of ten cases

Saxena,; Mishra, S. R.; Kashyap,; Choudhry,; Mahapatra,; Bhargava, Manorama

doi:10.1046/j.1365-2516.2000.00372.x

Cited by 51 publications

(54 citation statements)

References 33 publications

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“…Although this is considered an unusual finding in acquired hemophilia, in which most inhibitors show a type II kinetics and a small amount of residual FVIII activity may be detected, other case series with no baseline residual Factor VIII activity have been reported in the literature. 18 In the few cases with inhibitors showing a type II kinetics described thus far, 11 treatment with rituximab resulted in complete responses with the same patterns as in our series.…”

Section: Discussionsupporting

confidence: 82%

Selective B-cell depletion with rituximab for the treatment of patients with acquired hemophilia

Stasi

Brunetti

Stipa

et al. 2004

Blood

197

170

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The activity and safety profile of selective B-cell depletion with rituximab, an anti-CD20 monoclonal antibody, were evaluated in 10 patients with acquired hemophilia. Rituximab was given intravenously at the dose of 375 mg/m 2 once weekly for 4 consecutive weeks. Infusion-related side effects were observed in 3 patients but were of mild intensity and did not require discontinuation of treatment. Eight patients with Factor VIII (FVIII) inhibitor titers between 4 and 96 Bethesda units per milliliter (BU/mL) achieved a complete remission, which was defined as a return to normal FVIII activity and undetectable FVIII inhibitor titers. Two more patients with inhibitor levels greater than 100 BU/mL experienced only a partial transient decrease of the inhibitor after rituximab alone, but they achieved a complete response after being challenged with a combination of rituximab plus pulse intravenous cyclophosphamide. With a median follow-up of 28.5 months (range, 12-41 months), 3 patients have thus far relapsed. Retreatment with the monoclonal antibody at the same dose and schedule resulted in a new sustained response in all these patients. In conclusion, rituximab appears an effective and well-tolerated treatment for patients with acquired hemophilia and low inhibitor titers. A reinforcement of therapy with other agents seems to be required to achieve a full and durable response in those patients with high inhibitor levels. (Blood. 2004;103: 4424-4428)

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Section: Discussionsupporting

confidence: 82%

Selective B-cell depletion with rituximab for the treatment of patients with acquired hemophilia

Stasi

Brunetti

Stipa

et al. 2004

Blood

197

170

View full text Add to dashboard Cite

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“…The clinical characteristics of the disease have been previously described. [1][2][3] The literature describing acquired hemophilia A is based on tertiary referral single-center cohorts, [4][5][6][7][8][9][10][11][12][13][14][15][16] larger retrospective surveys of referral center patients, 1,5,[17][18][19] and patients reported because they have been treated for bleeding. [20][21][22][23] These reports have been combined in a review and meta-analysis.…”

Section: Introductionmentioning

confidence: 99%

“…22,23 Data on inhibitor eradication, however, are based on relatively small, uncontrolled single-center cohorts [4][5][6][7][8][9][10][11][12][13][14][15][16] and a metaanalysis. 24 These reports are potentially unrepresentative because referral center patients may be a subgroup of patients.…”

Section: Introductionmentioning

confidence: 99%

Acquired hemophilia A in the United Kingdom: a 2-year national surveillance study by the United Kingdom Haemophilia Centre Doctors' Organisation

Collins

Hirsch

Baglin

et al. 2006

Blood

664

1,037

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Acquired hemophilia A is a severe bleeding disorder caused by an autoantibody to factor VIII. Previous reports have focused on referral center patients and it is unclear whether these findings are generally applicable. To improve understanding of the disease, a 2-year observational study was established to identify and characterize the presenting features and outcome of all patients with acquired hemophilia A in the United Kingdom. This allowed a consecutive cohort of patients, unbiased by referral or reporting practice, to be studied. A total of 172 patients with a median age of 78 years were identified, an incidence of 1.48/million/y. The cohort was significantly older than previously reported series, but bleeding manifestations and underlying diseases were similar. Bleeding was the cause of death in 9% of the cohort and remained a risk until the inhibitor had been eradicated. There was no difference in inhibitor eradication or mortality between patients treated with steroids alone and a combination of steroids and cytotoxic agents. Relapse of the inhibitor was observed in 20% of the patients who had attained first complete remission. The data provide the most complete description of acquired hemophilia A available and are applicable to patients presenting to all

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“…The first aim of treatment is to control bleeding, which is typically accomplished by using either human factor VIII concentrate or porcine factor VIII (1, 3, 6, 13). Factor VIII inhibitor bypassing agent (FEIBA) and factor VII, which bypasses the VIII system, are replacement therapies used for severe bleeding or high levels of inhibitors in the patient (14). Steroid therapy is also a primary modality to treat and manage inhibitors.…”

Section: Discussionmentioning

confidence: 99%

Rupture of the Gallbladder in a Patient with Acquired Factor VIII Inhibitors and Systemic Lupus Erythematosus

et al. 2004

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A 54-year-old woman with a 21-year history of systemic lupus erythematosus (SLE) was admitted to the Matsuyama Red Cross Hospital due to subcutaneous and gingival hemorrhaging. She was diagnosed with acquired factor VIII inhibitors based on a prolonged activated partial-thromboplastin time (APTT) and factor VIII inhibitors. Steroid pulse and factor VIII plasma concentrate were administered to her, not long after which she was transferred to Ehime University Hospital due to gallbladder hematoma. Although her APTT and factor VIII activity were improved after treatment with human factor VIII, she died of multiple organ failure. The autopsy demonstrated a ruptured gallbladder.

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Acquired Haemophilia – a study of ten cases

Cited by 51 publications

References 33 publications

Selective B-cell depletion with rituximab for the treatment of patients with acquired hemophilia

Selective B-cell depletion with rituximab for the treatment of patients with acquired hemophilia

Acquired hemophilia A in the United Kingdom: a 2-year national surveillance study by the United Kingdom Haemophilia Centre Doctors' Organisation

Rupture of the Gallbladder in a Patient with Acquired Factor VIII Inhibitors and Systemic Lupus Erythematosus

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